Rationale and methodology of a collaborative learning project in congenital cardiac care.

BACKGROUND: Collaborative learning is a technique through which individuals or teams learn together by capitalizing on one another's knowledge, skills, resources, experience, and ideas. Clinicians providing congenital cardiac care may benefit from collaborative learning given the complexity of the patient population and team approach to patient care. RATIONALE AND DEVELOPMENT: Industrial system engineers first performed broad-based time-motion and process analyses of congenital cardiac care programs at 5 Pediatric Heart Network core centers. Rotating multidisciplinary team site visits to each center were completed to facilitate deep learning and information exchange. Through monthly conference calls and an in-person meeting, we determined that duration of mechanical ventilation following infant cardiac surgery was one key variation that could impact a number of clinical outcomes. This was underscored by one participating center's practice of early extubation in the majority of its patients. A consensus clinical practice guideline using collaborative learning was developed and implemented by multidisciplinary teams from the same 5 centers. The 1-year prospective initiative was completed in May 2015, and data analysis is under way. CONCLUSION: Collaborative learning that uses multidisciplinary team site visits and information sharing allows for rapid structured fact-finding and dissemination of expertise among institutions. System modeling and machine learning approaches objectively identify and prioritize focused areas for guideline development. The collaborative learning framework can potentially be applied to other components of congenital cardiac care and provide a complement to randomized clinical trials as a method to rapidly inform and improve the care of children with congenital heart disease.

Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest.

OBJECTIVES: To identify the clinical presentation of children and adolescents affected by 1 of 4 cardiac conditions predisposing to sudden cardiac arrest: hypertrophic cardiomyopathy, long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and anomalous origin of the left coronary artery from the right sinus of Valsalva (ALCA-R). STUDY DESIGN: This was a retrospective review of newly diagnosed pediatric patients with hypertrophic cardiomyopathy, LQTS, CPVT, and ALCA-R referred for cardiac evaluation at 6 US centers from 2008 to 2014. RESULTS: A total of 450 patients (257 male/193 female; median age 10.1 years [3.6-13.8 years, 25th-75th percentiles]) were enrolled. Patient age was ≤13 years for 70.4% of the cohort (n = 317). Sudden cardiac arrest was the initial presentation in 7%; others were referred on the basis of abnormal or suspicious family history, personal symptoms, or physical findings. Patients with LQTS and hypertrophic cardiomyopathy were referred most commonly because of family history concerns. ALCA-R was most likely to have abnormal signs or symptoms (eg, exercise chest pain, syncope, or sudden cardiac arrest). Patients with CPVT had a high incidence of syncope and the greatest incidence of sudden cardiac arrest (45%); 77% exhibited exercise syncope or sudden cardiac arrest. This study demonstrated that suspicious or known family history plays a role in identification of many patients ultimately affected by 1 of the 3 genetic disorders (hypertrophic cardiomyopathy, LQTS, CPVT). CONCLUSION: Important patient and family history and physical examination findings may allow medical providers to identify many pediatric patients affected by 4 cardiac disorders predisposing to sudden cardiac arrest.

Oral treprostinil use in children: a multicenter, observational experience.

Pulmonary arterial hypertension is a progressive, incurable disease that occurs in adults and children alike. Therapeutic options for children are limited and infrequently described, including newer agents such as treprostinil, an oral prostanoid. Herein, we describe the pooled pediatric experience in 28 patients from four pediatric pulmonary hypertension programs over two years. This descriptive, observational study describes the various methods of initiation of oral treprostinil in both prostanoid-naïve patients and those transitioning from parenteral or inhaled prostanoids. The youngest patient was four years old and the smallest weighed 16 kg. We describe adverse reactions and their management. Most patients in this study (27/28) were able to successfully initiate therapy. However, gastrointestinal adverse reactions were common; half of the patients started on this therapy had discontinued it within the two-year study period.

Inhaled treprostinil via the Tyvaso Inhalation System through a tracheostomy.

A 20-year-old man with pulmonary arterial hypertension secondary to systemic sclerosis was admitted to our hospital. Prior to admission, his PAH had been successfully managed with the use of tadalafil, ambrisentan and inhaled Tyvaso. Owing to respiratory failure from vocal cord paralysis, he underwent an emergent tracheotomy. The delivery of inhaled Tyvaso through a tracheostomy tube was explored. Post-tracheostomy, the patient continued his ability to self-administer the medication. His WHO functional classification, brain natriuretic peptide levels, and echocardiograms were not significantly different when Tyvaso was administered via tracheostomy compared with oral administration. This case report summarises the method used to deliver Tyvaso via a tracheostomy tube, which proved to be successful in this patient.

Low-dose nitric oxide therapy for persistent pulmonary hypertension: 1-year follow-up.

BACKGROUND: Although inhaled nitric oxide has been shown to reduce the use of extracorporeal membrane oxygenation, there is limited data on its effect on long-term outcomes. OBJECTIVE: The purpose of our study is to report on the 1 year outcome of neonates treated with inhaled nitric oxide compared to a group of neonates who did not receive nitric oxide. METHODS: We have previously reported on the in-hospital outcomes of 248 neonates who were >34 weeks' gestational age and were randomized to receive low-dose inhaled nitric oxide or placebo. Extracorporeal membrane oxygenation was used in 78 (64%) neonates in the control group and in 48 (38%) neonates in the inhaled nitric oxide group (p=0.001). We now report on the outcome of neonates followed to 1 year of age. RESULTS: Of the 248 neonates twenty-four (10%) died before 1 year of age. There was no difference in mortality between the two groups (11% in the control group and 9% in the inhaled nitric oxide group). Of the 224 surviving infants, we were able to contact the parents or guardians of 201 (90%) children. There were no intergroup differences in the numbers of patients reported as requiring medications for pulmonary disease (14% in the control group and 14% in the inhaled nitric oxide group) or the need for supplemental oxygen (1% in the control group and 0% in the inhaled nitric oxide group). The number of neonates reported to have an abnormal neurological examination or developmental delay was also similar in both groups (14% in the control group and 19% in the inhaled nitric oxide group). CONCLUSIONS: The use of low-dose inhaled nitric oxide reduces the use of extracorporeal membrane oxygenation without increasing the incidence of adverse outcomes to 1 year of age.

Impact of early fundoplication or gastrostomy tube on midterm outcomes for patients with single ventricle.

OBJECTIVE: Patients with single-ventricle heart disease experience early and late failure during and after staged palliation. Little is known about the factors related to continued risk of transplantation and mortality after completion of staged palliation. The long-term outcome of patients with single-ventricle disease who require a gastric fundoplication early in life has not been assessed. METHODS: A total of 155 patients with single-ventricle disease who survived their first-stage palliative procedure were enrolled in a research registry. Demographic and anatomic variables were collected, and the families were contacted every 6 months for prospective documentation of transplant-free survival. Medical records were reviewed for the details of noncardiac surgical procedures. Univariate and multivariate regression analyses were performed to determine the impact of early gastric surgery on late transplant-free survival. RESULTS: There were 93 male patients, median gestational age was 38 weeks, and birth weight was 3.2 kg. Sixty-five patients (42%) had hypoplastic left heart syndrome. Twelve patients (7.7%) had a genetic syndrome. Thirty-two patients (21%) had a fundoplication or a gastrostomy tube at less than 2 years of age. Median follow-up was 4.3 years (range, 79 days to 10 years). Race, gender, gestational age, birth weight, and genetic syndrome did not alter midterm transplant-free survival. Need for fundoplication or gastrostomy was an independent risk factor for decreased transplant-free survival (P = .003; hazard ratio, 4.29), which was unchanged when adjusted for all covariates. CONCLUSIONS: The need for early fundoplication or gastrostomy is associated with decreased transplant-free survival for patients with palliated single-ventricle heart disease.

Does ablation of supraventricular tachycardia in children with a structurally normal heart improve quality of life?

INTRODUCTION: Ablation has become first-line therapy for managing many pediatric patients with atrioventicular accessory pathway or atrioventricular nodal-mediated supraventricular tachycardia (SVT). Studies to date have all focused on elimination of the SVT substrate; yet, there are no studies to document whether this results in improved quality of life (QoL) scores. OBJECTIVE: The objective of this study was to evaluate whether a successful catheter ablation improved QoL scores in pediatric patients. PATIENT AND METHODS: Pediatric patients between the age of 5 and 18 years with structurally normal hearts and re-entry SVT that were referred to the electrophysiology laboratory between October 2004 and June 2006 at the Children's Healthcare of Atlanta were eligible to be enrolled in the study. The Pediatric Quality of Life Inventory cardiac model questionnaire was administered to patients prior to and 6 months following catheter ablation. Areas evaluated were physical, emotional, social, school, and psychosocial function. The paired t-test was used to test the difference between pre- and post-time points for the study groups. P value <.05 was considered significant. RESULTS: Forty-three patients consented to initial enrollment and returned the initial questionnaires. Seven patients did not have ablations performed during the electrophysiology study and therefore were excluded from further analysis. Complete pre- and post-ablation data were available for 27 patients. Comparing pre-ablation data with post-ablation data, there was significant improvement in all measured areas following successful elimination of tachycardia substrate. Patients reported lowest social and physical functioning scores pre-ablation. The greatest reported improvement post-ablation was in physical functioning. CONCLUSION: Successful elimination of SVT substrate results in improved pediatric QoL scores as reported by patients.

Pre- and postoperative quantitation of right ventricular tissue Doppler velocities in infants with hypoplastic left heart syndrome.

BACKGROUND: The purpose of this study was to use tissue Doppler imaging (TDI) to serially quantitate initial and preoperative right ventricular (RV) TDI velocities and compare them to postoperative RV TDI velocities as measures of RV function in newborns with hypoplastic left heart syndrome (HLHS). METHODS: Twelve consecutive patients were prospectively studied with diagnosis of HLHS. Systolic (Sw) and early diastolic (Ew) velocities were recorded at the tricuspid annulus and the ventricular septum at (1) admission, (2) immediately preoperative, and (3) during recovery. All patients were treated preoperatively with prostaglandins (PGE). Velocities were compared using repeated measure analysis of variance. RESULTS: Mean age at diagnosis was 1 day (0-4 days). Time from diagnosis to surgery was 4 days (1-9 days), age at surgery 5.3 days (2-10 days), and time from surgery to postoperative echo 12.3 days (5-19 days). Tricuspid annular and septal systolic velocities increased from admission to preoperative. Both tricuspid Sw and Ew and septal Sw velocities decreased postoperatively. No significant changes occurred in the ventricular septal diastolic (Ew) velocities from admission to preoperative. CONCLUSION: These data indicate that following the institution of PGE and initial medical and ventilatory management, there is an increase in RV annular and septal systolic velocities from the initial to the preoperative period and that these indices as well as RV annular diastolic velocities decline significantly postoperatively. Initial septal diastolic velocities were severely abnormal and did not significantly change pre- and postoperatively. These data may have significance for both postoperative and subsequent long-term RV function.